Results 31 to 40 of about 8,300 (285)

THERPA v2: an update of a small molecule database related to prion protein regulation and prion disease progression [PDF]

Prion, 2019
Prion diseases are rare, rapidly progressive neurodegenerative disorders that affect mammalian species [1,2].
Sol Moe Lee, Sung Soon Kim, Heebal Kim, Su Yeon Kim   +3 more
openaire   +2 more sources

Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]

, 2013
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
Jeremy C. Simpson (29225), Franke, Markus, Sibeal Waldron (277507), Appelhans Dietmar, Rogers Mark S., Dietmar Appelhans (277509), Resenberger, Ulrike K., Simpson Jeremy C., Simpson, Jeremy C., Jörg Tatzelt, Franke Markus, Resenberger Ulrike K., Rogers, Mark S., McCarthy James M., Mark S. Rogers (277510), James M McCarthy, Sibeal Waldron, Dietmar Appelhans, Appelhans, Dietmar, Mark S Rogers, Tatzelt Jörg, Tatzelt, Jörg, Ulrike K. Resenberger (277506), McCarthy, James M., Waldron, Sibeal, Markus Franke, Ulrike K Resenberger, Markus Franke (277505), Waldron Sibeal, Jeremy C Simpson, Jörg Tatzelt (277508), James M. McCarthy (277504)   +31 more
core   +1 more source

Cryo-EM structure of disease-related prion fibrils provides insights into seeding barriers [PDF]

Nature Structural & Molecular Biology, 2021
ABSTRACT One of the least understood aspects of prion diseases is the structure of infectious prion protein aggregates. Here we report a high-resolution cryo-EM structure of amyloid fibrils formed by human prion protein with Y145Stop mutation that is associated with a familial prion disease. This structural insight allows us not only to
Qiuye Li, Christopher P. Jaroniec, Witold K. Surewicz   +2 more
openaire   +2 more sources

Transgenic Mice Expressing Porcine Prion Protein Resistant to Classical Scrapie but Susceptible to Sheep Bovine Spongiform Encephalopathy and Atypical Scrapie

Emerging Infectious Diseases, 2009
How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE)
Juan-Carlos Espinosa, María-Eugenia Herva, Olivier Andréoletti, Danielle Padilla, Caroline Lacroux, Hervé Cassard, Isabelle Lantier, Joaquin Castilla, Juan-María Torres   +8 more
doaj   +1 more source

Increased Attack Rates and Decreased Incubation Periods in Raccoons with Chronic Wasting Disease Passaged through Meadow Voles

Emerging Infectious Diseases, 2022
Chronic wasting disease (CWD) is a naturally-occurring neurodegenerative disease of cervids. Raccoons (Procyon lotor) and meadow voles (Microtus pennsylvanicus) have previously been shown to be susceptible to the CWD agent.
S. Jo Moore, Christina M. Carlson, Jay R. Schneider, Christopher J. Johnson, Justin J. Greenlee   +4 more
doaj   +1 more source

Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

Journal of Neurology, Neurosurgery & Psychiatry, 2018
ObjectiveTo assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD).MethodsWe examined medical records of 117 sCJDVV2 (ataxic type), 65 sCJDMV2K (kuru-plaque type) and 121 sCJDMM(V)1 (myoclonic type) subjects for clinical symptoms, objective signs and ...
Baiardi S., Redaelli V., Ripellino P., Rossi M., Franceschini A., Moggio M., Sola P., Ladogana A., Fociani P., Magherini A., Capellari S., Giese A., Caughey B., Caroppo P., Parchi P.   +14 more
openaire   +3 more sources

Chronic Wasting Disease Prions in Elk Antler Velvet

Emerging Infectious Diseases, 2009
Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the ...
Rachel C. Angers, Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O’Rourke, Aru Balachandran, Glenn C. Telling   +8 more
doaj   +1 more source

The anti‐prion RNA aptamer R12 disrupts the Alzheimer's disease‐related complex between prion and amyloid β [PDF]

The FEBS Journal, 2019
The neurodegenerative disorder Alzheimer's disease (AD) is associated with the accumulation of misfolded proteins. Some recent studies suggested that amyloid beta (Aβ) forms soluble oligomers, protofibrils, and fibrils; the Aβ oligomers being more toxic than the fibrils.
Mamiko, Iida, Tsukasa, Mashima, Yudai, Yamaoki, Masatomo, So, Takashi, Nagata, Masato, Katahira   +5 more
openaire   +2 more sources

Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles.

, 2006
Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model.
Bari Michele A. Di, Boyle, Aileen, Lipp Hans-Peter, Gabriele Vaccari, Maurizio Pocchiari, Cardone Franco, Sbriccoli Marco, Claudia Cartoni, Sbriccoli, Marco, Boyle Aileen, Dell'Omo, Giacomo, Nonno, Romolo, Di Bari, Michele A, Nonno Romolo, Franco Cardone, Galeno, Roberta, Marco Sbriccoli, Paola Fazzi, Vaccari Gabriele, Cartoni, Claudia, Cardone, Franco, Galeno Roberta, Loredana Ingrosso, Roberta Galeno, Fazzi, Paola, Lipp, Hans-Peter, Fazzi Paola, Michele A Di Bari, Ingrosso Loredana, Hans-Peter Lipp, Ingrosso, Loredana, Pocchiari, Maurizio, Umberto Agrimi, Pocchiari Maurizio, Vaccari, Gabriele, Agrimi, Umberto, Giacomo Dell'Omo, Moira Bruce, Aileen Boyle, Dell'Omo Giacomo, Agrimi Umberto, Bruce Moira, Bruce, Moira, Romolo Nonno, Cartoni Claudia   +44 more
core   +1 more source

A Case–Control Study on the Origin of Atypical Scrapie in Sheep, France

Emerging Infectious Diseases, 2009
A matched case–control study (95 cases and 220 controls) was designed to study risk factors for atypical scrapie in sheep in France. We analyzed contacts with animals from other flocks, lambing and feeding practices, and exposure to toxic substances ...
Alexandre Fediaevsky, Eric Morignat, Christian Ducrot, Didier Calavas   +3 more
doaj   +1 more source